Cerebellar pleomorphic xanthoastrocytoma: case report and literature review.

Background: PXA generally has histologic features characteristic of benign biological behavior, although malignant forms have been reported. This neoplasm has also been observed in atypical locations.

Methods: The authors report a case of cerebellar PXA with rapid malignant transformation in a 58-year-old woman and review the rare presentations and atypical features of this tumor.

Results: Among the "unusual" locations, the most frequent is the cerebellum with 15 cases having been described, 9 in adults, with an average age of 33 years. In contrast, supratentorial forms had a younger age profile (26 years). The time from onset of symptoms to diagnosis was approximately 5.3 months. PXA in the posterior fossa had a higher rate of solid enhancing tumor (9/14). Regarding histologic appearance, two thirds were composite lesions.

Conclusions: The clinicopathologic features of cerebellar PXA show some differences from PXA located in the cerebral hemispheres. Recognizing the potential for PXA to present with unusual manifestations, regardless of location, has an obvious impact on the accuracy of diagnosis.